儿童侵袭性纤维瘤病的MRI表现与病理对照

Comparison of MRI Manifestations and Pathology with Aggressive Fibromatosis in Children

目的 分析侵袭性纤维瘤病(AF)的MRI表现,并与病理结果对照,提高对儿童AF的影像诊断水平。方法 回顾性分析14例AF患儿的MRI表现,包括病灶的部位、大小、形态、信号及强化特点等,并与病理结果进行对照分析。结果 14例病灶均为单发,腹外型11例,腹壁型2例,腹内型1例。最大径3.4~10.5 cm,平均6.6 cm。类圆形5例,“蟹足状”生长9例。T1WI呈等/稍低信号。T2WI呈不均匀高信号,内可见条片状低信号。8例可见血管流空信号。所有病变均未见出血、坏死成分。12例扩散加权成像(DWI)均呈高信号,平均表观扩散系数(ADC)值为1.41×10^(-3)mm^(2)/s。13例增强后均呈明显强化,其中11例病灶中可见“反转强化”。10例复查MRI可见肿瘤复发。镜下主要由梭形纤维母细胞/肌纤维母细胞和胶原纤维组成。免疫组织化学显示14例Vimentin、SMA、β-catenin均呈阳性,CD34均呈阴性。结论 儿童AF的MRI表现具有一定的特征性,如T2WI病变中条片状低信号、无明显扩散受限及“反转强化”等,可反映肿瘤的病理学特征。

Objective To analyze the MRI manifestations of aggressive fibromatosis(AF),compare with pathological results,and improve the imaging diagnosis of AF in children.Methods The MRI manifestations of 14 children with AF were analyzed retrospectively,including the location,size,shape,signal and enhancement features of the lesions,and compared with the pathological results.Results All the 14 lesions were solitary,extra abdominal type in 11 cases,abdominal wall type in 2 cases and intra-abdominal type in 1 case.The maximum diameter of the lesion was 3.4-10.5 cm,with an average of 6.6 cm.Five cases showed quasi-round shape and 9 cases showed“crab foot”growth.T 1WI showed iso or slightly low signal intensity.T 2WI showed uneven high signal intensity with strip low signal intensity in it.Vascular flow void signal was seen in 8 cases.No bleeding or necrotic components were found in all lesions.All the 12 cases of DWI showed high signal intensity,and the average ADC value was 1.41×10^(-3)mm^(2)/s.Thirteen cases showed obvious enhancement on contrast-enhanced scan,and“inversion enhancement”could be seen in 11 cases.Tumor recurrence was found in 10 cases by MRI.Under microscope,it was mainly composed of spindle fibroblasts,fibrocytes and collagen fibers.Immunohistochemistry showed that 14 cases were positive for Vimentin,SMA,β-catenin,and negative for CD34 protein.Conclusion The MRI manifestations of children's AF have certain characteristics,such as strip low signal intensity on T 2WI,no obvious diffusion limitation and“inversion enhancement”etc,which can reflect the pathological features of the tumor.